Quality of Life of Patients with Wilson's Disease and Their Families.

Objectives: Wilson's disease (WD) is a chronic disease caused by altered copper metabolism requiring lifelong therapy. Its long-term and debilitating nature has the potential to affect the quality of life (Qol) of patients as well as their families. Our study aims to assess this impact of the disease on patients and their families. Methods: We conducted a prospective, observational study over 2 years on 73 patients and 73 age-matched controls with 33 children and 40 adults in each group. The Qol of cases and controls was assessed using the PedsQL Generic Core Scales and World Health Organisation Quality of Life BREF (WHOQOL-BREF) for children and adults, respectively. Families of child and adult patients were interviewed using PedsQL Family Impact Module and Family Attitude Scale (FAS), respectively. The data were statistically analyzed. Results: Mean age of the cases was 22.04 ± 11.8 years. Qol scores for both adults and children were worse in cases with neuropsychiatric disease than in those with hepatic disease. For children, the mean scores of overall psychological functioning were lower in cases compared with controls (P = 0.0001). Qol of parents of the patients was significantly lower than those of parents of the controls as was the family functioning (P = 0.0001 and P = 0.016). Family Attitude Scale scores for adults did not differ significantly between cases and controls. Conclusion: The Qol of patients with neuro-WD is worse than that of hepatic disease. The disease impacts the psychological functioning of the children and the Qol of their families, which improves with the duration of the disease. What is known: WD is a long-term, debilitating disease. Patients have to take lifelong treatment with frequent medical visits and often multiple hospitalizations. What is new: WD affects the Qol of not only the patients but also their families. Qol of patients with neuro-WD is worse than that of patients with hepatic disease.

Umbilical venous blood gas analysis for neonatal assessment.

Umbilical venous pH is claimed to mirror fetal acid-base status. Importantly, it is easier to perform. The aim of this study is to : 1. Evaluate the umbilical venous blood gas (UVBG) profile of normal newborns and to compare UVBG in the following situations: presence or absence of asphyxia presence or absence of meconium-stained amniotic fluid presence or absence of respiratory distress: and in newborns that did and did not survive and 2. To study the peripheral venous blood gas (PVBG) profile of newborns that develop cardio-respiratory insufficiency (CRI). Venous blood gas (VBG) was performed in 961 newborns consecutively admitted to the neonatal nursery over one year and in 100 controls. PVBG was also performed in 370 admissions to the nursery that developed signs of CRI. Demographic, clinical and outcome data were collected. Mean UVBG parameters (pH, pCO2, HCO3, base deficit, pO2 and SO2%) were compared between different groups of newborns using t test. This study had ethical approval. Of the 3349 live births over one year, 961 (28.7%) needed intensive care. Of these, 123 (12.8%) died. All the mean cord gas parameters differed significantly between who died and who survived. Metabolic acidosis and hypoxemia were conspicuous on PVBG among babies with CRI. Except for PO2 all the mean parameters of UVBG differed significantly with asphyxia and meconium-stained amniotic fluid. When similar comparison was made among newborns that did or that did not develop respiratory distress, the difference was significant for all the parameters except SO2%. UVBG gives useful information about common neonatal morbidities and appears to be a useful tool for neonatal assessment. PVBG gives objective information about babies with CRI that may need special interventions.

Syndromic management of common illnesses in hospitalized children and neonates: a cost identification study.

OBJECTIVE: To find out drug treatment cost per illness per patient admitted to pediatric ward. METHODS: Patients admitted to pediatric ward over a period of 1 year were studied without exclusions. Following presentations were studied: fever, rapid breathing, diarrhea, severe malnutrition and neurological problems such as altered conscious level or convulsion. In this prospective observational study, patients with other problems were excluded. The subjects were also categorized as critically sick, sick and stable. Expenditure on medicines was calculated individually for each patient. Total expenditure, average cost and illness-wise cost were subsequently derived. Management of illnesses was on the lines of existing guidelines of our center. Sick newborns or newborns referred for special care were separately studied. Following outcome variables were studied: death or discharge, length of hospital stay and the day on which symptomatic relief was noted. RESULTS: 774 children and 141 newborns were studied. 25(3.2%) died. Presenting features were as follows: fever-568 (73.4%), rapid breathing-175 (22.6%), diarrhea-145 (18.7%), mild-moderate malnutrition-278 (35.8%), severe malnutrition-111 (14.3%) and neurological problems-41 (5.3%). Category-wise distribution was as follows: critically sick-89 (11.3%), sick-188 (24.3%) and stable-497 (46.2%). Average hospital stay was 7.1 days and symptomatic relief was experienced by day three in 77.7% cases. Average cost of medicines per patient was INR-167.8 (USD-4.2), 173 patients required oxygen and mean expenditure on oxygen was INR-310 (USD-8) and 68 patients required inotropes with a mean expenditure of INR-198 (USD-5). Of the 141 newborns admitted, 20(14.1%) died. Mean hospital stay was 9.8 days and average cost of drug treatment was INR-790 (USD-20) in newborns. CONCLUSIONS: This cost analysis study presents drug treatment costs for common illnesses at a referral centre in a developing country. It gives an option to choose drugs for an optimum mix of cost and effectiveness.

Declining seroprevalence of HIV infection among paediatric inpatients.

An unlinked anonymous testing for HIV infection among consecutive paediatric admissions at Cama and Albless Hospital, Mumbai, was performed in 1998-99 and again in 2003-04. The seroprevalence declined from 5.5% (24/535) to 2.2% (10/447). Greater awareness, enhanced antenatal detection and measures taken to prevent mother-to-child transmission (MTCT) seem to have contributed to the declining seroprevalence. We believe that paediatric inpatients reflect the extent of MTCT in the community.

Sustainable decline in mortality among severely malnourished children with local resources.

Sustainability of survival among malnourished children is a challenge. Over a period of four years, we achieved a significant decline in mortality, using locally available food, medicines and simple protocols.

Leptospirosis in children.

Leptospirosis has a broad spectrum of clinical manifestations varying, from inapparent influenza like illness to fulminant fatal disease with hepato-renal dysfunction and hemorrhagic phenomena. Our cases had fever, puffiness, respiratory distress and bleeding diathesis as leading manifestations. Leptospirosis was suspected in view of epidemic situation prevailing in the city. We report four cases here, three of which survived and one died.